eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Coarctation of the Aorta

Author: P Syamasundar Rao, MD, Professor of Pediatrics and Medicine, University of Texas-Houston Medical School; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas
Coauthor(s): Paul M Seib, MD, Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital
Contributor Information and Disclosures

Updated: Dec 20, 2006

Introduction

Background

Coarctation of the aorta (CoA) is a relatively common defect that accounts for 5-8% of all congenital heart defects. CoA may occur as an isolated defect or in association with various other lesions, most commonly bicuspid aortic valve and ventricular septal defect (VSD). The diagnosis of CoA may be missed unless an index of suspicion is maintained, and diagnosis is often delayed until the patient develops congestive heart failure (CHF), which is common in infants, or hypertension, which is common in older children. This article discusses the pathology, pathophysiology, clinical features, noninvasive and invasive evaluation, and therapy in patients with CoA.

Pathologic anatomy

CoA may be defined as a constricted aortic segment that comprises localized medial thickening with some infolding of the medial and superimposed neointimal tissue. The localized constriction may form a shelflike structure with an eccentric opening or may be a membranous curtainlike structure with a central or eccentric opening. The coarctation may be discrete, or a long segment of the aorta may be narrowed; the former is more common.

In the past, CoA has been described as preductal (or infantile) type or postductal (or adult) type, depending on whether the coarctation segment is proximal or distal to the ductus arteriosus, respectively. However, a closer examination of the anatomy suggests that all coarctations are juxtaductal.

The classic CoA is located in the thoracic aorta distal to the origin of the left subclavian artery at about the level of the ductal structure. However, rarely, a coarcted segment is present in the lower thoracic or abdominal aorta. In such instances, the coarcted segment may be long and fusiform with irregular lumen; many consider these to be inflammatory or autoimmune in origin, and they may be variants of Takayasu arteritis.

Dilatation of the descending aorta immediately distal to the coarctation segment (poststenotic dilatation) is usually present. A jet lesion on the wall of the aorta distal to the coarctation site may also be present. Varying degrees of hypoplasia of the isthmus of the aorta (the portion of the aorta between the origin of the left subclavian artery and ductus arteriosus) are present in most patients with thoracic coarctation; this hypoplasia may be significant in symptomatic coarctation of the neonate and infant, while, in children and adults, the isthmus may have only mild narrowing. The transverse aortic arch (the arch between the origin of the right innominate artery and the left subclavian artery) is also hypoplastic in symptomatic neonates and infants. Collateral vessels that connect arteries from the upper part of the body to the vessels below the level of coarctation may be seen; these may be present as early as a few weeks to a few months of life.

The most commonly associated clinically significant defects include patent ductus arteriosus, ventricular septal defect, and aortic stenosis. The earlier the infant presents, the more likely a significant associated defect is present. Bicuspid aortic valve may be seen in nearly two thirds of infants with CoA, while only 30% of those infants have an older sibling with such an anomaly.

Mitral valve anomalies, although less common than those of the aortic valve, are also associated with CoA. Sometimes, CoA is a complicating feature of a more complex cyanotic heart defect, such as transposition of the great arteries, Taussig-Bing anomaly, double-inlet left ventricle, tricuspid atresia with transposition of the great arteries, and hypoplastic left heart syndrome. Aortic coarctation is extremely rare in patients with severe right ventricular outflow tract obstructions such as tetralogy of Fallot and pulmonary atresia with intact ventricular septum. Some patients with CoA may have cerebral aneurysms, predisposing them to cerebrovascular accidents with severe hypertension later in life. CoA is the most common cardiac defect associated with Turner syndrome.

Pathogenesis

The exact mechanism by which aortic coarctation is produced is not clearly understood. The most commonly invoked hypotheses include hemodynamic and ectopic ductal tissue theories. In the hemodynamic theory, an abnormal preductal flow or abnormal angle between the ductus and aorta that increases right-to-left ductal flow and decreases isthmic flow potentiates development of coarctation. Postnatal spontaneous closure of the ductus arteriosus completes the development of aortic obstruction.

A high incidence of CoA in patients with congenital heart defects and decreased antegrade aortic flow in utero and virtual absence of CoA in patients with right heart obstructions would lend credence to the hemodynamic theory. Abnormal extension of ductal tissue into the aorta (ectopic ductal tissue) has been postulated to create the coarctation shelf and, with ductal closure, development of aortic obstruction. This theory, however, does not explain the variable degrees of isthmus and aortic arch hypoplasia associated with CoA.

Pathophysiology

CoA imposes significant afterload on the left ventricle (LV), which results in increased wall stress and compensatory ventricular hypertrophy.

The afterload may be imposed acutely, as occurs following closure of the ductus arteriosus in neonates with severe coarctation. These infants may rapidly develop CHF and shock. Rapid constriction of the ductus arteriosus, producing sudden severe aortic obstruction, seems to be the most likely explanation. As the ductus (aortic end) constricts, the left ventricular afterload rapidly increases, with a resultant increase in left ventricular pressures (systolic and diastolic). This causes elevation of the left atrial pressure, which may open the foramen ovale, causing left-to-right shunt and dilatation of the right atrium and right ventricle. If the foramen ovale does not open, pulmonary venous pressures and pulmonary artery pressures increase, and right ventricular dilatation develops. Cardiomegaly revealed by chest roentgenography and right ventricular hypertrophy seen on ECG and echocardiography are related to the indirect effects of rapid development of severe aortic obstruction.

LV afterload may also increase gradually, allowing children with less severe coarctation to develop arterial collateral vessels that partially bypass the aortic obstruction. These children may be asymptomatic until hypertension is detected or another complication develops.

The mechanism for development of hypertension is not clearly understood; mechanical obstruction and renin-angiotensin–mediated humoral mechanisms have been postulated.

The mechanical obstruction theory explains the increased blood pressure by postulating that a higher blood pressure is required to maintain flow through the coarcted segment and collateral vessels. The stroke volume, ejected into the limited aortic receptacle, produces a higher pressure proximal to coarctation. However, this theory does not explain the following:

  • The lack of relationship between the degree of elevation of blood pressure and the magnitude of obstruction
  • The increased peripheral vascular resistance distal to the site of obstruction
  • The delayed or lack of reduction of blood pressure immediately following relief of obstruction

The humoral theory postulates activation of the renin-angiotensin system secondary to reduction of renal blood flow and appears to explain most of the clinical features. However, measurement of plasma renin activity in both animal models and human subjects did not show consistently elevated plasma renin levels in the early studies. The reasons for the inability to demonstrate elevation of renin levels may be related to inadequate measurement of salt intake, posture, extracellular fluid volume, and sympathetic influences on renin release. More recent studies demonstrated abnormalities in renin-angiotensin-aldosterone systems. In addition, activation of central sympathetic nervous system may also be responsible for hypertension of aortic coarctation.

Associated anomalies greatly influence pathophysiology. VSD often coexists, and coarctation exacerbates the associated left-to-right shunt. Other levels of left heart obstruction (aortic stenosis, subaortic stenosis) may be present and may add to LV afterload.

A number of neurohumoral changes occur with CHF. Sympathetic nervous system activation occurs, resulting in increases in heart rate and blood pressure (BP). The renin-angiotensin system is activated in patients with CHF, particularly in CoA in which lower-body BP and renal perfusion may be reduced. Activation of the renin-angiotensin system results in vasoconstriction, cell hypertrophy, and the release of aldosterone. The role of the renin-angiotensin system in CHF and the use of drugs to modulate this system are an intense area of research. Unlike most cases of CHF, CoA is more complex because precoarctation and postcoarctation hemodynamics are quite different.

Drugs typically used to treat patients with CHF, such as angiotensin-converting enzyme inhibitors and, more recently, angiotensin II antagonists, may have adverse effects in patients with CoA. Attempts to achieve a normal precoarctation BP with these drugs may result in inadequate lower-body perfusion and may precipitate renal failure.

Vasopressin is also increased in heart failure, although its major stimulus for release is angiotensin II. Vasopressin affects free water retention and may result in hyponatremia. The vasoconstrictive properties of vasopressin may further elevate BP in coarctation.

Other substances, such as human brain natriuretic peptide (BNP), an endothelin, may be activated by CHF, although their specific role in coarctation has not been studied.

An additional cause of CoA is trauma that results in aortic dissection. Compromise of the true lumen of the aorta can result in the clinical picture of coarctation with reduced lower-extremity pulses. Urgent intervention is required in this circumstance.

Frequency

United States

CoA is a common defect and occurs in 6-8% of patients with congenital heart disease. However, coarctation may be found more frequently in infants who present with symptoms prior to age one year.

International

The prevalence of CoA appears to be lower (<2%) in Asian countries than in European and North American countries.

Mortality/Morbidity

Past autopsy studies suggest that the mortality rate in patients in whom CoA is not repaired surgically is 90% by age 50 years, with a mean age of 35 years. In the current era, CoA mortality is often determined by patient age, patient size, and associated major cardiovascular anomalies.

Associated problems that may contribute to death or morbidity include hypertension, intracranial hemorrhage, aortic rupture or dissection, endocarditis, and CHF.

Race

No definitive racial differences have been documented in CoA, although some authors have suggested that CoA is less common in Asians.

Sex

The male-to-female ratio is 2:1, although this ratio is not valid in abdominal CoA, in which this rare lesion predominantly affects females. The ratio of abdominal-to-thoracic coarctation is approximately 1:1000. The male preponderance observed in older patients is not seen in infants with CoA.

Age

Generally, patients with CoA present early in life with CHF or later in life with hypertension. Studies continue to document that CoA is often missed in the first year of life, and the median age of referral to a pediatric cardiologist in one study was 5 years. Among 2192 patients reported to the Pediatric Cardiac Care Consortium from 1985-1993, 1337 were infants, 824 were children, and 31 were adults.

Clinical

History

The presentation of patients with coarctation of the aorta (CoA) varies but may be discussed relative to the patients who present early, often with congestive heart failure (CHF), and the patients who present later, most often with hypertension.

  • Early presentation: The presence of associated defects and aortic arch anomalies, the extent of patency of the ductus arteriosus, the rapidity of the process of closure of the ductus arteriosus, and the level of pulmonary vascular resistance determine the timing of clinical presentation and the severity of symptoms. Young patients may present in the first few weeks of life with poor feeding, tachypnea, and lethargy and progress to overt CHF and shock. These patients may have appeared well prior to hospital discharge, and deterioration coincides with closure of the patent ductus arteriosus (PDA). Presentation may be abrupt and acute with ductal closure. Development of symptoms is often accelerated by the presence of associated major cardiac anomalies, such as ventricular septal defect (VSD). Symptoms may be subtle at first, and patients may make repeated trips to the physician before finally presenting in extremis.
  • Late presentation: Patients often present after the neonatal period with hypertension or a murmur. These patients often have not developed overt CHF because of the presence of arterial collateral vessels. Diagnosis is often made after hypertension is noted as an incidental finding during evaluation of other problems, such as trauma or more routine illness. Other presenting symptoms may include headaches, chest pain, fatigue, or even life-threatening intracranial hemorrhage. True claudication is rare, although an occasional child may experience pain or weakness in the legs. Many patients are asymptomatic except for the incidentally noted hypertension. Frequently, CoA is not recognized by the primary care physician. Palpation of femoral pulses and measurement of blood pressure during routine examination is necessary to avoid a delay in the diagnosis.

Physical

As with history, physical examination may conveniently separate patients into 2 groups: those who present early with heart failure and those who present later with hypertension.

  • Early presentation
    • Neonates may be found to have tachypnea, tachycardia, and increased work of breathing and may even be moribund with shock. Keys to the diagnosis include blood pressure (BP) discrepancies between the upper and lower extremities and reduced or absent lower extremity pulses to palpation. However, when the infant is in severe heart failure, all pulses are diminished. Upon treatment for heart failure, prominent brachial pulses with weak or nonpalpable femoral arterial pulses may be discerned. Diminished pulses on examination should never be disregarded, since the digits appreciate the rate of change in BP, which may be diminished in ductally dependent coarctation, although the measured BP may not show discrepancies. In patients with an aberrant origin of the right subclavian artery from the aorta distal to the obstruction, such discrepancies in BP may not be present, although lower extremity pulses are diminished versus the carotid pulses.
    • Differential cyanosis (pink upper extremities with cyanotic lower extremities) may occur when right-to-left shunt across a PDA provides flow to the lower body. Although often not obvious to the eye, differential cyanosis may be documented based on preductal and postductal pulse oximetry measurements and careful inspection. However, in the presence of lesions with large left-to-right shunt (eg, VSD), pulmonary artery saturations may approximate aortic saturations with less obvious differential oximetric findings. Reversed differential cyanosis (upper body cyanosis with normal lower-body saturation) may occur with transposition of the great arteries, PDA, and pulmonary hypertension, resulting in right-to-left ductal shunting.
    • In low cardiac output and ventricular dysfunction, pulses may be diminished diffusely, and BP gradients may seem minimal. Thus, in addition to coarctation, the differential diagnosis of perinatal circulatory insufficiency always includes left ventricular (LV) outflow obstruction, including aortic valve stenosis, subaortic stenosis, and supravalvar aortic stenosis, as well as severe mitral stenosis or insufficiency.
    • The murmur associated with CoA may be nonspecific yet is usually a systolic murmur in the left infraclavicular area and under the left scapula. Additional murmurs that result from the presence of associated abnormalities, such as VSD or aortic valve stenosis, may also be detected. An ejection click may signify the presence of a bicuspid aortic valve, while a gallop rhythm may indicate ventricular dysfunction.
  • Late presentation
    • Older infants and children may be referred for evaluation of hypertension or murmur. Hypertension in a fussy infant or a child may be attributed to agitation; thus, comparing 4 BP readings in the extremities is important. Occasionally, the left arm pressure is lower than the right arm pressure if the origin of the left subclavian artery is involved in the coarctation. Similarly, anomalous origin of the right subclavian artery (below the level of coarctation) may produce decreased or absent right brachial pulse. Careful simultaneous palpation of upper and lower extremity pulses may help confirm suspected coarctation.
    • In older children, adolescents, and adults, CoA is best diagnosed clinically based on simultaneous palpation of femoral and brachial pulses. Blood pressure in both arms and one leg must be determined; a pressure difference of more than 20 mm Hg in favor of the arms may be considered evidence of CoA.
    • A murmur in the left infraclavicular area and under the left scapula may be systolic, but the murmur may also sound continuous in the presence of multiple collateral vessels or, occasionally, severe coarctation. An ejection click may be audible when an associated bicuspid aortic valve and a murmur of aortic stenosis or insufficiency are present. Similarly, a murmur of mitral stenosis or LV outflow tract obstruction may also occur. A gallop rhythm may occur in the presence of a hypertrophic noncompliant LV.
    • Other findings on physical examination may include abnormalities of blood vessels in the retina and a prominent suprasternal notch pulsation. A thrill may be present in the suprasternal notch or on the precordium in the presence of significant aortic valve stenosis. In the rare case of abdominal coarctation, an abdominal bruit may be noted.

Causes

A number of theories exist regarding the etiology of CoA, including postnatal ductal constriction, translocation of ductal tissue onto the aorta, and a theory that alterations in intrauterine blood flow cause altered flow through the aortic arch and result in the substrate for coarctation. CoA manifests when the ductus closes starting at the pulmonary end, with gradual involution of ductal tissue toward the aorta.

Similar to most forms of congenital heart disease (CHD), the etiology of CoA may be explained by multifactorial inheritance hypothesis. The prevalence of CoA in genetic abnormalities such as Turner syndrome (45,X), is as high as 15-20%. Familial patterns of inheritance of coarctation have been reported, as well as for other left heart obstructive lesions. An increase in seasonal occurrence of CoA is reported in September and November.

More on Coarctation of the Aorta

Overview: Coarctation of the Aorta
Differential Diagnoses & Workup: Coarctation of the Aorta
Treatment & Medication: Coarctation of the Aorta
Follow-up: Coarctation of the Aorta
References
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Further Reading

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Keywords

coarctation of the aorta, CoA, left ventricular obstruction, LV outflow tract obstruction, aortic arch obstruction, interrupted aortic arch, left ventricular hypertrophy, left ventricular afterload, aortic obstruction, bicuspid aortic valve, ventricular septal defect, VSD, left heart obstruction, aortic stenosis, heart defects, congenital heart disease, congestive heart failure, CHF, hypertension, perinatal circulatory insufficiency

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Contributor Information and Disclosures

Author

P Syamasundar Rao, MD, Professor of Pediatrics and Medicine, University of Texas-Houston Medical School; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas
P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, American Pediatric Society, Medical Association of Georgia, Society for Cardiac Angiography and Interventions, Society for Pediatric Research, Southern Society for Pediatric Research, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Coauthor(s)

Paul M Seib, MD, Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital
Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Medical Editor

Juan Carlos Alejos, MD, Associate Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California at Los Angeles
Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation
Disclosure: Actelion Honoraria Speaking and teaching

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Julian M Stewart, MD, PhD, Director of Center for Pediatric Hypotension, Professor, Departments of Pediatrics and Physiology, Division of Pediatric Cardiology, Westchester Medical Center and New York Medical College
Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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